progressive supranuclear palsy (Steele-Richardson-Olszewski syndome)

• about 8% of patients with clinical diagnosis of Parkinson disease
• median age of onset = 64 yrs (range 50-77)

• core clinical syndrome
  • supranuclear ophthalmoplegia
  • akinesia (presents as frequent falls)
  • rigidity
  • nuchal dystonia
  • pseudobulbar palsy (dysarthria, dysphagia)
  • cognitive abnormality leading to dementia

• imaging
  • atrophy of midbrain (substantia nigra) and pontine tegmentum with abnormally enlarged prepontine cistern
  • variable atrophy of globus pallidus
  • some cases show atrophy of fronto-temporal regions with enlarged lateral ventricles and third ventricle
  • some cases show atrophy of motor cortex

• neuropathological changes
  • neuronal loss and gliosis with neurofibrillary tangles
    • oculomotor nucleus
    • substantia nigra
    • basal ganglia

• DDx
  • cortico-basal degeneration
  • multiple system atrophy
    • striatonigral degeneration
    • Shy-Drager syndrome
    • olivo-ponto-cerebellar atrophy
  • arteriosclerotic disease with lacunar infarction
  • idiopathic Parkinson disease

© 2002, C.E. Kahn, Jr.
2002-04-18